Idiopathic guttate hypomelanosis is a common acquired leukoderma characterized by multiple, discrete round or oval, porcelain-white macules on sun-exposed areas, especially on the extensor surface of forearms and pretibial areas. It usually affects individuals aged over 40 years and the likelihood of acquiring it increases with age. The exact pathogenesis remains controversial. However, there are several factors that are believed to be involved such as aging, ultraviolet exposure, trauma, genetic factors, autoimmunity, and local inhibition of melanogenesis. Despite the benign course of progression, many patients visit medical centers owing to cosmetic concerns and to confirm the natural course of idiopathic guttate hypomelanosis. Because there is no standard therapy for this condition, numerous medical and surgical treatments including intralesional corticosteroids, topical retinoids, topical calcineurin inhibitors, phenol peeling, cryotherapy, superficial dermabrasion, skin grafting, and ablative and non-ablative lasers have been tested with mixed results. This article will thoroughly review the etiology, pathogenesis, clinical presentations, histologic, dermoscopic, and ultrastructural findings, and the treatment of idiopathic guttate hypomelanosis.